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What is pulmonary
fibrosis?

Highlights

Pulmonary fibrosis is a condition that causes lung scarring and stiffness.

The cause is often unknown.

No cure is available.

Pulmonary fibrosis is a condition that causes lung scarring
and stiffness. This makes it difficult to breathe. It can prevent your body
from getting enough oxygen and eventually lead to respiratory failure, heart
failure, or other complications.

Autoimmune diseases, exposure to certain chemicals, and
genetics can raise your risk of developing pulmonary fibrosis. But in many
cases, the exact cause of the condition is unknown.

What
are the symptoms of pulmonary fibrosis?

You can have pulmonary fibrosis for some time without any
symptoms. Shortness of breath is typically the first symptom that develops.
Other symptoms can include:

a chronic, dry, and hacking cough

weakness

fatigue

curving of the fingernails, which is called clubbing

weight loss

a rapid heart rate

Because the condition generally affects older adults, early symptoms are
often misattributed to age or a lack of exercise. Your symptoms may seem minor
at first and progress over time. But they can vary from one person to another.
Some people with pulmonary fibrosis become ill very quickly.

What causes
pulmonary fibrosis?

The causes of pulmonary fibrosis can be divided into four categories:

autoimmune diseases

chemical exposure

unknown causes

genetics

Autoimmune diseases

Autoimmune diseases cause your body’s immune system to
attack itself. Autoimmune conditions that can lead to pulmonary fibrosis include:

rheumatoid arthritis

lupus erythematosus, which is commonly known as lupus

scleroderma

Churg-Strauss syndrome

polymyositis

dermatomyositis

polyangiitis

Chemical exposure

Exposure to chemicals can also contribute to pulmonary
fibrosis. For example, cigarette smoke contains many chemicals that can damage
your lungs and lead to this condition. Other things that can damage your lungs
include:

asbestos fibers

grain dust

silica dust

certain gases

radiation

Some medications may also raise your risk of developing
pulmonary fibrosis. If you have a family history of the condition, you should
speak with your doctor before taking medications that can damage your lungs,
including certain:

Unknown causes

In many cases, the cause of pulmonary fibrosis in unknown.
When this is the case, the condition is called idiopathic pulmonary fibrosis
(IPF). According to the National Heart, Lung, and Blood Institute, most
people with pulmonary fibrosis have IPF.

Genetics

About 10 to 15 percent of people with IPF have another
family member with pulmonary fibrosis, reports the Pulmonary Fibrosis Foundation. In these cases,
it’s known as familial pulmonary fibrosis or familial interstitial pneumonia.
Researchers have linked some genes to the condition, but more research about
what role genetics plays is necessary.

Who is at risk of
pulmonary fibrosis?

You’re more likely to be diagnosed with pulmonary fibrosis if you:

are male

are between the ages of 40 and 70

have a history of smoking

have a family history of the condition

have an autoimmune disorder associated with the
condition

have taken certain medications associated with the
disease

have undergone cancer treatments, particularly chest
radiation

work in an occupation associated with increased risk,
such as mining, farming, or construction

How is pulmonary fibrosis diagnosed?

Pulmonary fibrosis is one of more than 200 types of lung
diseases that exist. Because so many different lung diseases exist, your doctor
may have difficulty identifying that IPF is the cause of your symptoms. In a
survey by the Pulmonary Fibrosis Foundation, 55 percent of
respondents reported being misdiagnosed at some point. The most common
misdiagnoses were asthma, pneumonia, and bronchitis.

The most definitive way to diagnose pulmonary fibrosis is by
taking a tissue sample. Your doctor can take a tissue sample using bronchoscopy.
In this procedure, they’ll pass a small tube through your mouth to your lungs.
Then, they’ll pass a small brush, forceps, or a needle through the tube to
collect a small sample of tissue.

If they need a larger tissue sample, your doctor might
perform a surgical biopsy. They’ll make an incision in your chest to access
your lung tissue and collect a sample. They can examine the biopsied tissue to
check for signs of pulmonary fibrosis and determine how far your condition has
progressed.

Your doctor may also use a variety of other tools to
diagnose pulmonary fibrosis or rule out other conditions. These may include:

imaging scans, such as a chest X-ray, CT scan,
or echocardiogram

blood tests to assess the oxygen levels in your
blood

a sputum sample to check for signs of infection

a spirometry test to measure your lung capacity

a treadmill stress test to learn how your lungs
respond to physical activity

How
is pulmonary fibrosis treated?

No cure is available for pulmonary fibrosis. Your doctor
can’t reverse the lung scarring, but they can prescribe treatments to help
improve your breathing and slow the progression of the disease.

For example, your doctor may prescribe one or more
medications, such as:

prednisone to suppress your immune system and
reduce inflammation

methotrexate or cyclosporine to suppress your
immune system

N-acetylcysteine, which is an antioxidant that
doctors often prescribe along with other drugs to help slow the progression of
the disease

pirfenidone, which is an antifibrotic drug that
can help reduce inflammation and scarring in the lungs

Your doctor may also recommend oxygen therapy to help you breathe and sleep
more easily. They’ll ask you to wear a plastic tube in each nostril to receive
oxygen from a tank.

They may also recommend pulmonary rehabilitation. This treatment involves a
program of exercise, education, and support to help you learn how to breathe
more easily.

Your doctor may also encourage you to make changes to your lifestyle. These
changes may include the following:

You should avoid secondhand smoke, and you
should take steps to quit if you smoke. This can help slow the disease’s
progression and ease your breathing.

Eat a well-balanced diet. Your doctor,
nutritionist, or dietitian may recommend eating certain foods to help you
maintain a healthy weight.

Follow an exercise plan developed with your
doctor’s guidance. Exercise may help improve your lung function, strengthen
your heart, and strengthen your muscles, which can help you maintain your
energy levels.

Get adequate rest and avoid excess stress. Doing
these things may help improve your breathing.

Younger people who don’t respond to other treatments might
need a lung transplant.

What
is the outlook for people with pulmonary fibrosis?

The rate at which pulmonary fibrosis scars people’s lungs
varies. The scarring isn’t reversible, but your doctor can recommend treatments
to reduce the rate at which your condition progresses.

Many people with pulmonary fibrosis only live for about
three to five years after they’ve been diagnosed, reports the American Lung Association. The condition can
cause a number of complications, including respiratory failure. This happens
when your lungs no longer work properly and they can’t get enough oxygen to
your blood.

Pulmonary fibrosis also raises your risk of lung cancer.

Tips
for prevention

Some cases of pulmonary fibrosis may not be preventable.
Other cases are linked to environmental and behavioral risk factors that can be
controlled. Follow these tips to lower your risk of getting the disease:

Avoid smoking.

Avoid secondhand smoke.

Wear a face mask or other breathing device if
you work in an environment with harmful chemicals.

If you’re having trouble breathing, make an appointment with
your doctor. Early diagnosis and treatment can improve the long-term outlook
for people with many lung diseases, including pulmonary fibrosis. While the
disease can’t be cured, treatment may help slow its progression.